Progressive muscular atrophy (PMA)
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Progressive muscular atrophy (PMA). Males are more frequently affected. Fully referenced neurology checklist.
Demographic features
- Males are more frequently affected
- The onset age is older than in amyotrophic lateral sclerosis (ALS)
- About a fifth of patients develop upper motor neurone signs within 5 years
Genetic risk factors
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Pathology
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Clinical features
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Differential diagnosis
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References
- Kim W-K, Liu J, Sandner J, et al. Study of 962 patients indicates progressive muscular atrophy is a form of ALS. Neurology2009; 73:1686-1692.
- Cervenakova L, Protas II, Hirano A, et al. Progressive muscular atrophy variant of familial amyotrophic lateral sclerosis (PMA/ALS). J Neurol Sci 2000; 177:124-130.
- Pereira Â, Tkachenko N, Fortuna AM, Alonso I, Cardoso M, Da Silva JD. An SPG7 mutation as a novel cause of monogenic progressive muscular atrophy. Neurol Sci 2023 (Online ahead of print).
- Riku Y, Atsuta N, Yoshida M, et al. Differential motor neuron involvement in progressive muscular atrophy: a comparative study with amyotrophic lateral sclerosis. BMJ Open 2014; 4:e005213.
- Ince PG, Evans J, Knopp M, et al. Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS. Neurology 2003; 60:1252-1258.
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